Rabbit anti-Human, Mouse SPR Polyclonal Antibody | anti-SPR antibody

SPR, CT (SPR, Sepiapterin reductase) (MaxLight 550)

Cat. Num. AAA6350758

• Gene Names: SPR; SDR38C1
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Purified by Protein A Affinity and Peptide Affinity Chromatography.
• Synonyms: SPR; Polyclonal Antibody; CT (SPR; Sepiapterin reductase) (MaxLight 550); Sepiapterin reductase; anti-SPR antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human SPR. Species crossreactivity: mouse
• Purity/Purification: Purified by Protein A Affinity and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight550.

• Applicable Applications for anti-SPR antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within aa231-260 in the C-terminal region of human SPR, UniProt Accession #P35270.
• Conjugate: MaxLight550
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight550 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of mouse liver tissue lysates (35ug/lane) usingMBS6004814. SPR (arrow) was detected usingMBS6004814.)

Related Product Information for anti-SPR antibody

SPR encodes an aldo-keto reductase that catalyzes the NADPH-dependent reduction of pteridine derivatives and is important in the biosynthesis of tetrahydrobiopterin (BH4). Mutations in this gene result in DOPA-responsive dystonia due to sepiaterin reductase deficiency. A pseudogene has been identified on chromosome 1.

Product Categories/Family for anti-SPR antibody

Antibodies; Enzymes; Reductase

References

Leu-Semenescu, S., et al. Sleep 33(3):307-314(2010). Schnetz-Boutaud, N.C., et al. Genes Brain Behav. 8(8):753-757(2009). Clot, F., et al. Brain 132 (PT 7), 1753-1763 (2009).

NCBI and Uniprot Product Information

• NCBI GI #: 4507185
• NCBI GeneID: 6697
• NCBI Accession #: NP_003115.1
• NCBI GenBank Nucleotide #: NM_003124.4
• UniProt Accession #: P35270; Q53GI9; Q9UBB1; A8K741; D6W5H2
• Molecular Weight: 28,048 Da
• NCBI Official Full Name: sepiapterin reductase
• NCBI Official Synonym Full Names: sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)
• NCBI Official Symbol: SPR
• NCBI Official Synonym Symbols: SDR38C1
• NCBI Protein Information: sepiapterin reductase; short chain dehydrogenase/reductase family 38C, member 1
• UniProt Protein Name: Sepiapterin reductase
• Protein Family: Sproutin
• UniProt Gene Name: SPR
• UniProt Synonym Gene Names: SPR
• UniProt Entry Name: SPRE_HUMAN

NCBI Description

This gene encodes an aldo-keto reductase that catalyzes the NADPH-dependent reduction of pteridine derivatives and is important in the biosynthesis of tetrahydrobiopterin (BH4). Mutations in this gene result in DOPA-responsive dystonia due to sepiaterin reductase deficiency. A pseudogene has been identified on chromosome 1. [provided by RefSeq, Jul 2008]

Uniprot Description

SPR: Catalyzes the final one or two reductions in tetra- hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin. Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD). In the majority of cases, patients manifest progressive psychomotor retardation, dystonia and spasticity. Cognitive anomalies are also often present. The disease is due to severe dopamine and serotonin deficiencies in the central nervous system caused by a defect in BH4 synthesis. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Belongs to the sepiapterin reductase family.

Protein type: Cofactor and Vitamin Metabolism - folate biosynthesis; EC 1.1.1.153; Oxidoreductase

Chromosomal Location of Human Ortholog: 2p14-p12

Cellular Component: nucleoplasm; mitochondrion; cytoplasm; cytosol

Molecular Function: aldo-keto reductase activity; sepiapterin reductase activity; NADP binding

Biological Process: L-phenylalanine metabolic process; tetrahydrobiopterin biosynthetic process; voluntary musculoskeletal movement; death; pteridine metabolic process; neuron morphogenesis during differentiation; regulation of multicellular organism growth; regulation of nitric-oxide synthase activity; nitric oxide biosynthetic process; norepinephrine metabolic process; dopamine metabolic process; nitric oxide metabolic process; serotonin metabolic process

Disease: Dystonia, Dopa-responsive, Due To Sepiapterin Reductase Deficiency